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Updated: Sep 8, 2025
Neuro-rehabilitation Approach for Sudden Sensorineural Hearing Loss
Published on: January 25, 2016
Audiological Findings in Children with PLA2G6-Associated Neurodegeneration
Valiyaparambath Purushothaman Vandana1, Jeevendra Kumar Darshini1, Bindu Parayil Sankaran2,3
1Department of Speech Pathology and Audiology, National Institute of Mental Health and Neurosciences (NIMHANS), Bengaluru, Karnataka, India.
Insights
Audiological findings in children with PLA2G6-associated neurodegeneration (INAD) revealed hearing loss and auditory neuropathy spectrum disorder. Further research is needed for aural rehabilitation recommendations.
Area of Science:
- Pediatric Neurology
- Neurodegenerative Diseases
- Audiology
Background:
- Limited understanding of audiological manifestations in PLA2G6-associated neurodegeneration.
- Infantile neuroaxonal dystrophy (INAD) is a rare genetic disorder affecting the nervous system.
Purpose of the Study:
- To investigate and characterize the audiological findings in a cohort of 13 children diagnosed with INAD.
- To identify specific hearing impairments associated with this condition.
Main Methods:
- Comprehensive audiological assessments were performed.
- Tests included tympanometry, distortion product otoacoustic emissions, impedance audiometry, and Brainstem Auditory Evoked Potentials (BAEPs).
Main Results:
- Six out of 13 children presented with sensorineural hearing loss.
- Four children were diagnosed with auditory neuropathy spectrum disorder.
- These findings highlight significant auditory involvement in INAD.
Conclusions:
- The study expands the known auditory findings in INAD patients.
- Further research on quality of life and cognitive aspects is necessary.
- Recommendations for aural rehabilitation require additional investigation.
Background:
Audiological manifestations of patients with PLA2G6-associated neurodegeneration are limited.
Objective:
To analyze the audiological findings in a cohort of 13 children with infantile neuroaxonal dystrophy (INAD).
Method:
Patients underwent a battery of audiological tests including tympanometry, distortion product otoacoustic emissions, impedance audiometry, and Brainstem Auditory Evoked Potentials (BAEPs).
Results:
Audiological studies of 13 children indicated sensorineural hearing loss in six children and auditory neuropathy spectrum disorder in four children.
Conclusion:
This study may extend the auditory findings for INAD. Additional studies on quality of life and cognitive-brain degeneration related to this disease are required before making appropriate recommendations for aural rehabilitation.
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